Ramsay Hunt Syndrome Type 2

Key Points at a Glance

Highlights of This Disease

• Ramsay Hunt syndrome type 2 (RHS) is herpes zoster of the ear caused by reactivation of varicella-zoster virus in the geniculate ganglion, with facial nerve palsy as the main feature.
• It accounts for about 12% of all facial nerve palsies and is the second most common cause of non-traumatic peripheral facial palsy after Bell’s palsy.
• The triad consists of severe ear pain, vesicular rash around the auricle, and facial nerve palsy, but 8–25% of cases present without vesicles (zoster sine herpete).
• When eyelid closure is incomplete, corneal protection is urgent, and ophthalmologic management is essential.
• Starting combination therapy with steroids and antiviral drugs within 72 hours of onset determines prognosis, achieving a complete recovery rate of 70–75%.
• If treatment begins after 7 days, complete resolution occurs in only 30% of cases.
• Patients with lymphopenia during chemotherapy or immunocompromised patients are at risk of severe disease and bilateral involvement.

1. What is Ramsay Hunt Syndrome Type 2?

Ramsay Hunt Syndrome Type 2 (RHS) is a syndrome caused by reactivation of varicella-zoster virus (VZV) in the geniculate ganglion. It is also called herpes zoster oticus. It was first described in 1907 by American neurologist James Ramsay Hunt. ³⁾⁴⁾

Epidemiology:

• Accounts for approximately 12% of all facial nerve paralysis.
• It is the second most common cause of non-traumatic peripheral facial nerve paralysis after Bell’s palsy. ⁷⁾
• About 16.7% of facial paralysis in children is due to RHS. The incidence is 2.7 per 100,000 (under 10 years old), with a peak between 6 and 15 years. ³⁾
• It occurs more frequently in the elderly, under stress, and in immunocompromised individuals. ⁴⁾

Original classification by Hunt (1908): ⁷⁾

• Herpes zoster oticus only
• Herpes zoster oticus + facial nerve palsy
• Herpes zoster oticus + facial nerve palsy + hyperacusis
• Herpes zoster oticus + facial nerve palsy + Ménière’s syndrome

In 1910, Hunt added pharyngeal and laryngeal herpes zoster.

Differentiation from Bell’s palsy: RHS is characterized by severe ear pain and vesicular rash around the auricle. It differs from Bell’s palsy in that vestibulocochlear symptoms (vertigo, tinnitus, hearing loss) are more prominent, and it involves multiple cranial neuropathies including taste disturbance in the anterior two-thirds of the tongue. ⁵⁾

Q How is Ramsay Hunt syndrome different from Bell's palsy?

A

RHS is caused by reactivation of the varicella-zoster virus, presenting with vesicular rash around the ear and severe ear pain. Vestibulocochlear symptoms such as dizziness, tinnitus, and hearing loss are prominent, and taste disturbance in the anterior two-thirds of the tongue also occurs. Bell’s palsy is of unknown cause (possibly involving HSV-1) without vesicles, and its prognosis is better than that of RHS.

2. Main Symptoms and Clinical Findings

Subjective Symptoms

• Ear pain (otalgia): Severe ear pain is often the first symptom, lasting 1–3 days. ³⁾
• Vesicular rash: Appears on the auricle, external auditory canal, and oral cavity. Prodromal pain precedes the appearance of vesicles.
• Hearing loss: Reported in 24% of children. ³⁾ Sensorineural hearing loss occurs in 10% of adults. ¹⁾
• Dizziness, nausea, vomiting: Due to vestibular nerve disorder. Vestibular symptoms are observed in 40% of cases. ¹⁾
• Tinnitus: Due to disorder of the vestibulocochlear nerve.
• Dysgeusia: Affects the anterior two-thirds of the tongue. ⁵⁾
• Facial weakness or drooping: Paralysis of the facial muscles on the affected side.
• Tearing and eye pain: Symptoms associated with incomplete eyelid closure.
• Prodromal symptoms: Neuropathic pain, headache, malaise, sleep disturbance. ⁴⁾

Clinical Findings (Findings Confirmed by Physician Examination)

• Vesicular exanthema of the auricle and external auditory canal: Observed in the concha, auricle, and external auditory canal.
• Facial nerve palsy: Lower motor neuron type. Evaluated using the House-Brackmann classification.

The grades of the House-Brackmann classification (6 grades from I to VI) are shown below.

Grade	Findings
I	Normal
II	Mild dysfunction
III	Moderate dysfunction
IV	Moderate to severe dysfunction
V	Severe dysfunction
VI	Complete paralysis

• Lagophthalmos: Leads to dry eye and lower corneal epithelial damage. Urgent ophthalmological management is required.
• Positive Bell’s phenomenon: Upward rotation of the eyeball when unable to close the eyes. ⁴⁾
• Zoster sine herpete (RHS without rash): Reported in 8–25% of patients with acute peripheral facial nerve palsy. Varicella-zoster virus infection is confirmed without vesicles. 14% develop vesicles after facial weakness onset. ¹⁾⁶⁾
• MRI findings: Contrast-enhanced T1-weighted MRI shows abnormal enhancement of the facial nerve. Positively correlated with facial nerve swelling. ²⁾⁸⁾

Q Is Ramsay Hunt syndrome possible even without vesicles?

A

Yes. As zoster sine herpete (RHS without rash), it is reported in 8–25% of patients with acute peripheral facial nerve palsy. Diagnosis is possible by confirming varicella-zoster virus infection through PCR or serological tests even without vesicles.

3. Causes and Risk Factors

Cause: Reactivation of varicella-zoster virus latent in the geniculate ganglion. After primary infection (chickenpox), the virus remains dormant in the dorsal root ganglia and reactivates and proliferates during immunosuppression.

Risk factors:

• Immunocompromised state: Chemotherapy, HIV, diabetes, etc. ²⁾
• Chemotherapy-induced lymphopenia: Dose-dense chemotherapy leads to grade 3–4 severe lymphopenia in 63% of cases. Delayed recovery of CD4+ T lymphocytes contributes to varicella-zoster virus reactivation. ⁵⁾
• Concurrent use of dexamethasone (antiemetic): Causes synergistic immunosuppression through T-cell selective depletion, suppression of IL-2 production, and Th1→Th2 shift. ⁵⁾
• Solid tumors: Approximately twice the risk of developing herpes zoster compared to the general population. ⁵⁾
• Advanced age and stress: Triggers for reactivation of varicella-zoster virus. ⁴⁾
• No history of varicella vaccination: Two doses at 4- to 8-week intervals are recommended for primary prevention.

Prevention and Daily Care

Primary prevention is possible by administering two doses of the varicella vaccine at 4- to 8-week intervals. However, cases of Ramsay Hunt syndrome have been reported even after vaccination (possibly due to reactivation of the vaccine strain or subclinical infection). If symptoms appear after vaccination, seek medical attention promptly. Individuals who are immunocompromised (e.g., undergoing chemotherapy, HIV-infected, diabetic) are at particularly high risk, so it is important to consult a doctor early if ear pain or facial weakness occurs.

Q If I receive the varicella vaccine, will I not develop Ramsay Hunt syndrome?

A

Cases of RHS occurring after vaccination have been reported. Possible causes include reactivation of the vaccine strain or reactivation of a subclinical infection (past wild-type varicella-zoster virus infection). Vaccination reduces the risk of onset but does not guarantee complete prevention.

4. Diagnosis and Testing Methods

Clinical diagnosis: Based on the triad (severe ear pain, vesicles on the auricle or oral mucosa, and facial nerve palsy). ⁷⁾

Tests for definitive diagnosis

• PCR testing of vesicle fluid: Detection of varicella-zoster virus DNA. The most reliable method.
• Direct fluorescent antibody (DFA) test: Sensitivity 90%, specificity 99%.
• Serological tests (IgM/IgG antibody titers): Recommended as routine testing for acute peripheral facial nerve palsy in children. ³⁾
• PCR of ear discharge or oropharyngeal swab: Useful for early diagnosis of zoster sine herpete. Varicella-zoster virus DNA can be detected in middle ear fluid. Oropharyngeal swab PCR is considered more useful than serology for early diagnosis of ZSH. ⁶⁾
• Tear fluid culture PCR: May be positive for varicella-zoster virus, but note false positives in 25–35% of Bell’s palsy patients.

Imaging and Physiological Tests

• Contrast-enhanced MRI: Shows abnormal enhancement of the facial nerve. Also used to evaluate CNS complications. ²⁾⁸⁾
• Head CT: Ancillary test when CNS complications are suspected.
• Electroneuronography (ENoG): Evaluation of facial nerve degeneration.
• Blink reflex test: R1, R2, and contralateral R2 disappear on the affected side. ⁸⁾
• Pure-tone audiometry: Evaluation of sensorineural hearing loss. ⁸⁾
• Lumbar puncture (CSF analysis): When CNS complications are suspected. Confirm lymphocytic pleocytosis and positive varicella-zoster virus PCR. ³⁾⁸⁾

Differential Diagnosis

• Bell’s palsy: The most important differential. Lacks vesicles, ear pain, and vestibulocochlear symptoms.
• Postherpetic neuralgia・Temporomandibular joint disorder・Trigeminal neuralgia
• Benign paroxysmal positional vertigo
• Malignant tumor: Must be excluded if accompanied by vocal cord paralysis (cranial nerve X palsy). ¹⁾

5. Standard Treatment

The basic principle of treatment is early initiation of steroids plus antiviral drugs within 72 hours of onset.

The timing of treatment initiation and recovery rate of facial nerve palsy are shown below.

Timing of treatment initiation	Complete resolution rate
Within 3 days	70–75%
3–7 days	Approximately 48%
Day 7 and later	Approximately 30%

Pharmacotherapy

Antiviral drugs:

• Oral acyclovir: 800 mg 4–5 times daily for 7–10 days. ⁷⁾
• Oral valacyclovir: 500–1,000 mg 3 times daily for 7 days. ¹⁾⁶⁾
• Intravenous acyclovir: For CNS infection, administer 10–15 mg/kg every 8 hours for 10–14 days. ³⁾⁸⁾ In chemotherapy-related RHS, a report used 750 mg/day intravenously. ⁵⁾
• There are reports showing no significant difference between oral and intravenous administration (comparison of 48 oral vs 32 intravenous cases). ⁴⁾

Corticosteroids:

• Prednisolone: 1 mg/kg/day for 5–7 days, then taper. ³⁾⁷⁾
• Tapering from prednisone 60 mg/day is also used. ⁶⁾⁷⁾
• Methylprednisolone: May be used as a first-line steroid. ²⁾
• Combination therapy with steroids + acyclovir has a higher complete recovery rate than steroids alone. ²⁾

Symptomatic treatment:

• Vertigo: Diazepam
• Neuropathic pain: Carbamazepine, Gabapentin ⁴⁾
• Pain: Aceclofenac + Paracetamol ⁷⁾
• Tricyclic antidepressant (amitriptyline): Used for mental stability and sleep improvement. ⁴⁾

Ophthalmic Management

If lagophthalmos is present, corneal protection is the top priority.

• Artificial tears: administered 4 times daily. ¹⁾
• Ophthalmic ointment/eye patch: for corneal protection at night. ¹⁾
• Eye closure taping. ²⁾
• Regular ophthalmic follow-up. ¹⁾

Surgical Treatment (for Persistent Lagophthalmos)

• Tarsorrhaphy
• Gold weight implantation
• Horizontal eyelid shortening (e.g., lateral tarsal strip procedure)
• Medialization thyroplasty: When vocal cord paralysis (cranial nerve X) is present. ¹⁾

Follow-up

Monitor facial nerve injury using the House-Brackmann classification. Follow-up is recommended at 2 weeks, 6 weeks, and 3 months after starting treatment.

Treatment Precautions

If more than 72 hours have passed since onset, the recovery rate decreases significantly. It is important to seek medical attention promptly if ear pain or facial weakness occurs. If eyelid closure is incomplete, corneal epithelial damage and ulcers can develop, so early ophthalmologic evaluation is necessary. In immunocompromised patients, extended antiviral therapy may be required.

Q When should treatment ideally be started?

A

Starting treatment within 72 hours of onset is most important. If started within 3 days, the complete resolution rate of facial nerve palsy reaches 70-75%, but if started after 7 days, it drops to about 30%.

6. Pathophysiology and Detailed Mechanism of Onset

Causative virus: Varicella-zoster virus (chickenpox/shingles virus, human herpesvirus type 3) is a DNA virus. ⁴⁾

Mechanism of reactivation: After primary infection (chickenpox), varicella-zoster virus remains latent in sensory ganglion cells of the geniculate ganglion. During immune suppression, it reactivates and proliferates, causing herpetic inflammatory lesions from the ganglion to the associated skin dermatome. ⁴⁾

Neuropathology: Histologically, perivascular, perineural, and intraneural round cell infiltration is observed within the facial nerve. ⁴⁾⁷⁾

Mechanisms of polyneuritis (three theories):

• Proximity theory: Because the eighth cranial nerve is close to the seventh cranial nerve at the cerebellopontine angle, hearing loss and vertigo occur.
• Vascular route (vasa vasorum) theory: The virus travels to adjacent cranial nerves via nutrient blood vessels.
• Anterograde spread theory: Varicella-zoster virus spreads anterogradely through synaptic transmission in brainstem reflex pathways.

Mechanism of CNS infection: ³⁾⁸⁾

• Acute varicella-zoster virus encephalitis, post-varicella-zoster virus cerebellitis, varicella-zoster virus vasculopathy
• Possible retrograde axonal transport from the geniculate ganglion into the intracranial space, followed by descending spread causing RHS
• Hematogenous dissemination or spread to the CNS via CSF pathways

---

7. Latest Research and Future Prospects (Research Stage Reports)

For Patients: Please Read Carefully

The following content is currently at the research stage or under clinical trial and is not standard treatment available at general hospitals. It is reference information for professionals regarding future medical developments.

Onset of RHS in Chemotherapy Patients

Kanaya et al. (2025) reported two cases of RHS that developed during dose-dense chemotherapy (ddAC-ddPTX). Onset occurred at nadir lymphocyte counts of 590/μL and 630/μL, respectively, and both patients had residual facial nerve dysfunction (Yanagihara 22/40 and 24/40) after treatment. Although the NCCN guidelines do not routinely recommend antiviral prophylaxis for solid tumor patients, strategies such as minimizing the use of dexamethasone for antiemetic purposes and substituting olanzapine have been proposed. ⁵⁾

Delayed recovery of CD4+ T lymphocytes after chemotherapy is thought to affect varicella-zoster virus control, and revision of prevention strategies in immunocompromised patients remains a challenge. ⁵⁾

Intratympanic steroid injection

A systematic review and meta-analysis by Fujiwara et al. (2022) suggested that intratympanic steroid injection in RHS patients may contribute to recovery promotion. ²⁾

It is noted as an add-on effect to standard oral/intravenous therapy, but currently it is not recommended in routine clinical practice.

RHS complicated by varicella-zoster virus meningitis in young immunocompetent individuals

Hwang et al. (2023) reported two cases of immunocompetent men aged 32 and 43 who developed varicella-zoster virus meningitis followed by RHS. RHS complicated by varicella-zoster virus meningitis in immunocompetent individuals is rare, and cerebrospinal fluid varicella-zoster virus PCR and serological tests are essential for diagnosis. ⁸⁾

Prognosis of polyneuritis cranialis type RHS

Gillette et al. (2023) analyzed 23 reported cases of RHS with vocal cord paralysis (cranial nerve X) and found that the complete recovery rate for RHS with multiple cranial nerve involvement was only 27.3%, significantly lower than the 67.7–82.9% for isolated RHS. ¹⁾

---

8. References

1. Gillette BT, Heilbronn CM. A Rare Case of Vocal Cord Paralysis in the Setting of Ramsay Hunt Syndrome. Cureus. 2023;15(3):e36027.
2. Sheik-Ali S, Jiang Y, Nasef H, Sproson E, Tuohy O. Bilateral sequential Ramsay Hunt syndrome in an immunocompromised adult: a rare entity. Ann R Coll Surg Engl. 2024;106:197-199.
3. Ahmed EY, Al Rawahi H, Al Amrani F, Al Masaoudi L, Al Yazidi L. Ramsay Hunt Syndrome Associated with Varicella-Zoster Virus Encephalitis in a Child. Sultan Qaboos Univ Med J. 2024;24(1):127-130.
4. Dhatrak VM, Mohod S, Shinde SB, Jadhav VV. Ramsay Hunt Syndrome: A Rare Complication of Herpes Zoster Infection With an Incidental Finding of Submandibular Hemangioma. Cureus. 2024;16(8):e66020.
5. Kanaya E, Matsui K, Urasaki A, et al. Ramsay-Hunt Syndrome in Patients Undergoing Dose-Dense Chemotherapy in the Perioperative Period of Breast Cancer: Two Case Reports. Cureus. 2025;17(12):e100372.
6. Nishizawa T, Ishikawa K, Matsuo T, et al. Atypical Ramsay Hunt syndrome (zoster sine herpete) with otitis media. J Gen Fam Med. 2021;22:344-346.
7. Ghezta NK, Bhardwaj Y, Ram R, Basi RN. Ramsay Hunt Syndrome: A diagnostic dilemma. Natl J Maxillofac Surg. 2022;13:S179-182.
8. Hwang YS, Kim YS, Shin BS, Kang HG. Two cases of Ramsay-Hunt syndrome following varicella zoster viral meningitis in young immunocompetent men: case reports. BMC Neurol. 2023;23:43.